VasculitisEvelyn Osio-Salido, MD, MSc, FPCP, FPRA

Associate Professor

Section of Rheumatology, UPCM

Nothing to disclose

Objectives

At the end of the SYMPOSIUM, we hope the attendee will be able to:

1. Discuss the importance of basic knowledge about the vasculitides.

2. Suspect the possibility of vasculitis among patients in daily practice.

3. Institute initial management, as necessary

Objectives

At the end of the LECTURE, we hope the attendee will be able to:

1. Define vasculitis and vasculopathies.

2. Classify vasculitides

3. Recognize common presenting manifestations

1.KEEP CALM.2.You do not need

to memorize these.

3.Remember the essentials.

Definition

Vasculopathy –ANY disease of blood vessels

● Degenerative

● Metabolic

● Clotting disorders

● Embolic

● Inflammatory

ATHEROSCLEROSIS VASCULITIS

Deposition of plaque, made up of fat, collagen, elastin, macrophages, platelets, etc.

Inflammation then scarring of vessel wall.

https://cdn.davidwolfe.com/wp-content/uploads/2017/10/vasculitis-FI-800x419.jpg

Systemic vasculitis

INFLAMMATION of blood vessels

Involve SINGLE organ; often MULTISYSTEMIC

KNOWN or UNKNOWN causes

UNCOMMON but often SERIOUS May lead to death, organ damage, poor QOL.

BETTER OUTCOME with treatment; (+) Relapses

HIGHER RISKS of infection, CVD, malignancy

Kidder D, et al. Medicine (2017) https://doi.org/10.1016/j.mpmed.2017.11.001

Inflammation• Granulomatous

– Giant cell arteritis (GCA)

– Takayasu arteritis (TAK)

– Granulomatosis with polyangiitis (GPA)

– Eosinophilic granulomatosiswith polyangiitis (EGPA)

• Mononuclear cells without granuloma – Kawasaki disease (KD)

• Fibrinoid necrosis– Polyarteritis nodosa (PAN)

• Combined perivascular infiltrates

– Behcet’s disease

• Leukocytoclasis- nuclear debris of neutrophils– Henoch- Schonlein purpura

Inflammation• Granulomatous

– Giant cell arteritis, Takayasuarteritis, Granulomatosis with polyangiitis (GPA)

• Mononuclear cells without granuloma – Kawasaki disease

• Fibrinoid necrosis– Polyarteritis nodosa

(PAN)

Combined perivascular infiltrates - Behcet’s disease

Leukocytoclasis- nuclear debris of neutrophils

- Henoch- Schonlein purpura

Inflammation• Granulomatous

– GCA, TAK, GPA, EGPA

• Mononuclear cells without granuloma - KD

• Fibrinoid necrosis- PAN

Combined perivascular infiltrates- Behcet’s disease

Leukocytoclasis- nuclear debris of neutrophils

- Henoch- Schonlein purpura (HSP)

Single organ or multisystem

• SINGLE organ

– Skin

– CNS

• Multisystem

– G P A

– Granulomatosiswith polyangiitis

https://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcR45AoXAWTSYFzXcfZGtmEaPZ-93g2kbQDFySb0rgEnBppOWORdhttps://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcQ90s72Evo9VihzioI8NVcsv1eRCGnn7NnU1Wz6Uccpto_ZcFoQ

https://encrypted-tbn0.gstatic.com/images?q=tbn:ANd9GcR45AoXAWTSYFzXcfZGtmEaPZ-93g2kbQDFySb0rgEnBppOWORd

ORGAN GPA MPA EGPA PAN

ENT +++ + ++ polyps --

Kidney ++ +++ + + HPN

Nervous System

++ + +++ +++

Lungs ++ nodules +(+) diffuse + transient --

Eyes ++ + (+) --GIT +(+) +(+) ++ +++

Heart + (+) ++ --

Skin ++ ++ ++ ++

Muscle/Joint

++ ++ ++ ++

ANCA PR3 MPO MPO negative

Known or unknown cause

• PRIMARY-

• SECONDARY-

1. Infection- Hepatitis B, C, Syphilis, Salmonella, TB, Candida, Aspergillus

2. Radiation

3. Drugs- penicillin, quinolone, NSAID, PTU

4. Cancer

5. Connective tissue disease- SLE, RA, sarcoidosis

ORGANS

• Single

• Multiple

CAUSE

• Primary

• Secondary

Vessel SIZE

• Large

• Medium

• Small

• Variable

Classification

http://www.vasculitis.org.nz/__data/assets/image/0005/27536/Vasculitis_Image_1.jpg

Kidder D, et al. Medicine (2017) https://doi.org/10.1016/j.mpmed.2017.11.001

Large

•TAK

•GCA

Medium

•PAN

•KD

Small

•ANCA GPA EGPA MPA

•IC HSP anti GBM Cryoglobulin

CLASSIFICATION ACCORDING TO VESSEL SIZE

VARIABLE VESSEL: Behcet’s disease Cogan’s syndrome

http://www.vasculitis.org.nz/__data/assets/image/0007/27538/Vasculitis_Image_3.jpg

PAINSKIN

NERVES

SKINKIDNEYSLUNGSEYES

Copyrights apply

HSP

CV

MPAGPA

EGPA

Uncommon

Variable incidence of vasculitis depending on:

• TYPE OF VASCULITIS

– GCA 20 per 100,000 vs AAV 20 per million

• GEOGRAPHY

– MPA Japan > UK- 18.2 versus 6.5 per million adults

GPA Japan < UK- 2.1 versus 14.3 per million adults

– West- HSP is most common in children

Asia- Kawasaki disease is most common

If it is rare, why do we need to know it?

Case of Robert

32 year old healthy male, married, employed, physically active

• Sore throat, odynophagia, fever, body aches

• Erythema at pharynx

WHAT IS YOUR DIAGNOSIS?

Case of Robert

32 year old healthy male, married, employed, physically active

• Sore throat, odynophagia, fever, body aches

• Erythema at pharynx

• No response to antibiotics, disappear after weeks

• Recurrent 1x a year or EOY for 7 years

• Pain at R calf with non-itchy papules; similar papules at chest

• Review of history: recurrent oral ulcers (single or multiple) lasting for 1-2 weeks since > 15 years ago

BEHCET’s DISEASEoral ulcers, genital ulcers, uveitis, folliculitis,

erythema nodosum, myositis

Why we need to know vasculitis

• Symptoms & signs are like those of common diseases (a mimic).

• Symptoms & signs may seem mild/innocuous.

• Early treatment is better.

When to suspect vasculitis

• Unexplained systemic signs and symptoms, multisystem

– Fever, weight loss, rash, Proteinuria + casts, High ESR/CRP

• Arterial occlusion and ischemia- Raynaud’s, gangrene, claudication, infarction, stroke, hypertension in young adults

• Skin lesions- splinter hemorrhages (nails), palpable purpura, erythema nodosum, livedo reticularis, urticaria

• GN, Intestinal angina, Mononeuritis multiplex, Headache

• Inflammatory eye disease, sudden visual loss/retinal vascular disease (no HPN/DM)

• Arthralgias/arthritis, myalgias

Mimics of vasculitis

• Bacterial endocarditis• Antiphospholipid antibody syndrome (APAS)• Viral infections- HIV, CMV, HBV, HCV• Cholesterol emboli syndrome• Hypertensive arteriopathy• Vasoconstrictor drugs (ergot poisoning)• Thoracic outlet syndrome• Sepsis, DIC• Sarcoidosis• Thrombotic thrombocytopenic purpura (TTP)• Lymphoma, hair cell leukemia

Summary: Vasculitis

• Uncommon diseases that may present with common symptoms

• Manifestations depend on size and location of blood vessel involved

• Need for thorough investigation by the physician and health care team.

Thank you for listening.

04. Which of these microbes cause vasculitis through immune complex deposition?

A. Hepatitis B

B. Mycobacterium tuberculosis

C. Salmonella

D. Syphilis

05. Which vasculitis occurs among children less than 5 years old?

A. Giant cell arteritis

B. Polyarteritis nodosa

C. Kawasaki disease

D. Takayasu arteritis

06. Which is the correct match?

A. GPA- asthma

B. EGPA-swollen ear lobe

C. Behcet- oral ulcer

D. Takayasu- blindness